L P Jiao1, J F Fan, Q Sun, Y Shen. 1. National Key Discipline of Pediatrics (Capital Medical University) Ministry of Education, Beijing Children's Hospital, Capital Medical University, Beijing, 100045, China.
Abstract
BACKGROUND: Good pasture syndrome (GPS) has been paid much attention recently for the dangerous illness and high mortality. OBJECTIVE: To investigate the efficiency of plasma exchange (PE) to treat Goodpasture syndrome (GPS) in children associated with Turner's syndrome. METHOD: We report a case of a 15 year old female with GPS and Turner's syndrome. The patient has intermittent fever and cough for 45 days and oliguria for 6 days. Turner's syndrome was determined through blood karyotype analysis, and GPS was diagnosed because the patient was negative for antinuclear antibodies and antineutrophil cytoplasmic antibodies (ANCA), but positive for anti-glomerular basement membrane (anti-GBM) antibodies (200 RU/ml). PE was carried out in combination with immunosuppression therapy. RESULTS: The results show PE treatment can efficiently decrease the levels of anti-GBM antibodies. The antibody levels were >200 RU/ml and 184 RU/ml before and after the first PE treatment, respectively. The removal efficiency were 40%, 47%, 42%, 54%, 52% for the fifth, sixth, seventh, eighth and ninth PE procedures, respectively. CONCLUSION: The therapy with PE, hemodialysis, pulse methylprednisolone followed by oral prednisone and cyclophosphamide greatly contributed to improvement of this patient's condition, and resolved the patient's pulmonary haemorrhage. All these results demonstrate that PE contributed efficiently to the treatment for GPS in children.
BACKGROUND:Good pasture syndrome (GPS) has been paid much attention recently for the dangerous illness and high mortality. OBJECTIVE: To investigate the efficiency of plasma exchange (PE) to treat Goodpasture syndrome (GPS) in children associated with Turner's syndrome. METHOD: We report a case of a 15 year old female with GPS and Turner's syndrome. The patient has intermittent fever and cough for 45 days and oliguria for 6 days. Turner's syndrome was determined through blood karyotype analysis, and GPS was diagnosed because the patient was negative for antinuclear antibodies and antineutrophil cytoplasmic antibodies (ANCA), but positive for anti-glomerular basement membrane (anti-GBM) antibodies (200 RU/ml). PE was carried out in combination with immunosuppression therapy. RESULTS: The results show PE treatment can efficiently decrease the levels of anti-GBM antibodies. The antibody levels were >200 RU/ml and 184 RU/ml before and after the first PE treatment, respectively. The removal efficiency were 40%, 47%, 42%, 54%, 52% for the fifth, sixth, seventh, eighth and ninth PE procedures, respectively. CONCLUSION: The therapy with PE, hemodialysis, pulse methylprednisolone followed by oral prednisone and cyclophosphamide greatly contributed to improvement of this patient's condition, and resolved the patient's pulmonary haemorrhage. All these results demonstrate that PE contributed efficiently to the treatment for GPS in children.
Authors: Zbigniew M Szczepiorkowski; Jeffrey L Winters; Nicholas Bandarenko; Haewon C Kim; Michael L Linenberger; Marisa B Marques; Ravindra Sarode; Joseph Schwartz; Robert Weinstein; Beth H Shaz Journal: J Clin Apher Date: 2010 Impact factor: 2.821
Authors: I J Simpson; P B Doak; L C Williams; H A Blacklock; R S Hill; C A Teague; P B Herdson; C B Wilson Journal: Am J Nephrol Date: 1982 Impact factor: 3.754